We present two cases, women of 21 and 60 years old, who presented with deep vein thrombosis. Both cases had retroperitoneal para-aortic and iliac lymph node enlargement without any malignancy or other predisposing thrombophilic factors. Investigations revealed tubercular aetiology of the lymph nodes causing venae caval obstruction.
- retroperitoneal lymph nodes
- deep vein thrombosis
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Deep vein thrombosis can occur due to venous flow obstruction caused by retroperitoneal lymphomas and malignant masses. Tubercular lymph node enlargement occurs frequently in the superior mediastinum and retroperitoneal regions but has not been reported as causing vena caval obstruction leading to deep vein thrombosis.
A 60-year-old woman presented with asymmetric painful swelling of both lower limbs of 15 days duration. There was no history of fever, anorexia, or weight loss, or any suggestion of cardiovascular or respiratory illness. There was no recent pelvic or orthopaedic surgery, no recent long journey, and no trauma. There was no history of leg swelling, diabetes mellitus or thrombotic stroke. On examination she was found to have bilateral lower limb oedema (pitting) with positive Homan's sign suggestive of deep vein thrombosis. There was a 2 × 3 cm mobile lymph node in the right supraclavicular fossa of the neck. She was mildly febrile (99°F), but pulse and blood pressure were normal. Cardiovascular, respiratory system and abdominal examinations were normal. A provisional diagnosis of lymphoma with deep vein thrombosis was made. Routine investigations revealed normal haemogram, renal functions, blood sugar and chest X-ray. Liver function tests were normal except for hypoalbuminuria (serum albumin 27 g/l). Abdominal ultrasound revealed multiple matted para-aortic enlarged lymph nodes. This was confirmed on abdominal computed tomography (CT) (figure 1). Duplex colour ultrasound scanning of the lower limbs confirmed bilateral iliofemoral thrombosis extending up to the inferior vena cava (IVC) with external compression of the IVC and iliac veins by the lymph nodes. Fine needle aspiration cytology of the supraclavicular lymph node revealed features of tubercular lymphadenitis. The patient was treated with low molecular weight heparin (parnaparin 3200 IU daily, subcutaneously), along with antitubercular drugs (RHZE). There was regression of fever, supraclavicular lymph node and venous thrombosis and leg oedema. However, the patient was lost to follow-up, as she returned to her village.
A 21-year-old woman presented with bilateral asymmetric leg oedema of one week duration. On questioning, she admitted to having low-grade fever, anorexia, weight loss, cough (non-productive) and ammenorrhoea for 3 months. There was no history of haemoptysis, chest pain, breathlessness, joint pains, photosensitive skin rash, or alopecia. She denied being on oral contraceptives and a pregnancy test prior to admission was negative. There was no history of abortions. There was no history of recurrent limb oedema, thrombotic stroke, or valvular heart disease. On examination she had mild fever (99°F). Her pulse and blood pressure were normal and there was bilateral pitting lower limb oedema with positive Homan's sign and dilated veins over the anterior thighs and lower abdomen with flow upwards. There was no peripheral lymphadenopathy. Cardiovascular examination revealed dull second left ICS with faint heart sounds. Chest and abdominal examinations were unremarkable. Her haemogram, platelets, renal functions, liver functions and blood sugar were within normal limits except for a raised erythrocyte sedimentation rate (65 mm/h). ELISA for human immunodeficiency virus was negative. Her chest X-ray (figure 2) was suggestive of pericardial effusion.
Echocardiogram revealed moderate pericardial effusion with a structurally normal underlying heart without any cardiac tamponade or sign of constrictive pericarditis. Duplex colour ultrasound scanning of lower limbs and abdomen revealed bilateral iliofemoral venous thrombosis extending up to the hepatic IVC with extensive para-aortic and iliac bifurcation lymph node enlargement causing venous compression of IVC and iliac veins. A small volume of ascitic fluid was also noticed. Differential diagnosis of disseminated tuberculosis or systemic lupus erythematosus with deep vein thrombosis was made. All three sputum smears were positive for acid-fast bacillae. Rheumatoid factor and antinuclear factor were negative. Tests for proteins C and S as well as antithrombin III deficiency and antiphospholipid were within normal limits. A diagnosis of disseminated tuberculosis with deep vein thrombosis was made and she was treated with antitubercular drugs (RHZE), oral steroids and low molecular weight heparin (parnaparin 3200 IU daily, subcutaneously). There was rapid resolution of leg oedema, and remission of constitutional symptoms and weight gain.
After one month, a sputum smear for acid-fast bacillae was negative and her repeat echocardiogram showed complete resolution of pericardial fluid. Abdominal ultrasound showed a reduction in size of the para-aortic lymph nodes. She is on follow-up with antitubercular drugs and warfarin and continues to show improvement in general well being with complete disappearance of leg oedema.
Deep venous thrombosis can be broadly due to three factors (boxFB1). Hereditary thrombophilias generally present as recurrent venous thrombosis with familial tendencies in patients below 45 years of age and often at unusual sites like hepatic, portal, mesenteric, renal, cerebral or retinal veins.1
Retroperitoneal para-aortic lymphadenopathy is commonly seen in lymphomas and tuberculosis. Discrete but large lymph node masses in lymphomas are well known to cause mechanical vena caval obstruction.2 However, tubercular lymph nodes are generally small, and have not been reported to cause mechanical venous obstruction. Tubercular lymph nodes may cause IVC thrombosis either by a large collective matted mass or due to minor obstruction in the presence of an underlying predisposed hereditary thrombophilic state, similar to that described in Cockett's syndrome (left iliac vein thrombosis due to compression by right common iliac artery), pregnancy, malignant disease, or pelvic surgery.
Neither of our patients had a history of recurrent venous thrombosis. Moreover, in the second case, protein C, S, antithrombin III levels and activated partial thromboplastin time (for lupus anticoagulant) were normal, thus eliminating a predisposed thrombophilic state. Hence, large matted tubercular lymph node masses is the probable explanation for the IVC and iliofemoral thrombosis in these patients.
In conclusion, deep vein thrombosis may be one of the atypical presentations of tuberculosis and the possibility of retroperitoneal tubercular lymphadenitis should be considered in cases of unexplained deep vein thrombosis.